Summary

Pemphigus encompasses a group of life-threatening autoimmune blistering disorders characterized by intraepithelial blister formation. The molecular basis for intraepithelial blister formation is the loss of adhesion between kerati-nocytes, called acantholysis, which is caused by auto-Ab against intercellular adhesion structures of epidermal keratinocytes. Clinically, PV is characterized by extensive bullae and erosions of the mucous membranes and also of the skin (in case that anti-Dsgl auto-Ab are also present). Patients with untreated PV are prone to infections, loss of body fluids and proteins and to weight loss due to painful oral and esophageal erosions. The prognosis of PV was fatal prior to introduction of systemic glucocorticoids and adjuvant immunosuppressive agents as the standard treatment. Several forms of pemphigus have been identified depending of the level of the intraepidermal split formation. In PV, the blisters are located in the suprabasal layer whereas in PF, a clinically less severe disease, the blisters occur within the upper layers of the epidermis. In PV and PF, the auto-Ab target the extracellular portions of

Dsg3 and Dsgl, respectively. Auto-Ab production in PV and PF is polyclonal and most auto-Ab are of the IgG4 subclass in acute onset or active disease. Patients in remission have mainly auto-Ab of the IgGl subtype. Evidence for the pathogenicity of these circulating auto-Ab is provided by the observation that 1) the activity of PV correlates with auto-Ab titers, 2) newborns of mothers with active PV temporarily exhibit blisters due to the diaplacentar transfer of maternal auto-Ab, and 3) pemphigus-like lesions are induced in neonatal mice by transfer of IgG from PV patients. The major therapeutic strategy in pemphigus is chronic immunosuppressive therapy with glucocorticosteroids in combination with immunosuppressive adjuvants.

References

Ahmed AR, Blose DA (1984) Pemphigus vegetans. Neumann type and Hallopeau type. Int J Dermatol 23: 135-141

Ahmed AR, Yunis EJ, Khatri K (1990) Major histocompatibility complex haplotype studies in Ashkenazi Jewish patients with pemphigus vulgaris. Proc Natl Acad Sci USA 87: 7658-7662

Ahmed AR, Wagner R, Khatri K (1991) Major histocompatibility complex haplotypes and class II genes in non-Jewish patients with pemphigus vulgaris. Proc Natl Acad Sci USA 88: 5056-5061

Ahmed AR, Mohimen A, Yunis AJ (1993) Linkage of pemphigus vulgaris antibody to the major histocompatibility complex in healthy relatives of patients. J Exp Med 177: 419-424

Amagai M, Klaus-Kovtun V, Stanley JR (1991) Auto-Ab against a novel epithelial cadherin in pemphigus vulgaris, a disease of cell adhesion. Cell 67: 869-877

Amagai M, Karpati S, Prussick R, Klaus-Kovtun V, Stanley JR (1992) Autoantibodies against the amino-terminal cadherin-like binding domain of pemphigus vulgaris antigen are pathogenic. J Clin Invest 90: 919-926

Amagai M, Hashimoto T, Shimizu N, Nishikawa T (1994) Absorption of pathogenic autoantibodies by the extracellular domain of pemphigus vulgaris antigen (Dsg 3) produced by baculovirus: J Clin Invest 94: 59-67

Amagai M, Nishikawa T, Shimizu N, Green KJ, Hashimoto T (1995) Antigen-specific immunoadsorption of pathogenic autoantibodies in pemphigus foliaceus. J Invest Dermatol 104: 895-901

Amagai M, Koch PJ, Nishikawa T, Stanley JR (1996) Pemphigus vulgaris antigen (desmog-lein 3) is localized in the lower epidermis, the site of blister formation in patients. J Invest Dermatol 106: 351-355

Amagai M, Nishikawa T, Nousari HC, Anhalt GJ, Hashimoto T (1998) Antibodies against desmoglein 3 (pemphigus vulgaris antigen) are present in sera from patients with paraneoplastic pemphigus and cause acantholysis in vivo in neonatal mice. J Clin Invest 102: 775-782

Amagai M, Tsunoda K, Suzuki H, Nishifuji K, Koyasu S, Nishikawa T (2000a) Use of autoantigen-knockout mice in developing an active autoimmune disease model for pemphigus. J Clin Invest. 105: 625-31

Amagai M, Matsuyoshi N, Wang ZH, Andl C, Stanley JR (2000b) Toxin in bullous impetigo and staphylococcal scalded skin syndrome targets desmoglein 1. Nature Med 6: 1275-1277

Anhalt, GJ, Labib KS, Vorhees JS, Beals TF, Diaz LA (1982) Induction of pemphigus in neonatal mice by passive transfer of IgG from patients with the disease. N Engl J Med 306: 1189-1192

Anhalt GJ, Kim S, Stanley JR (1990) Paraneoplastic pemphigus: an autoimmune mucocutaneous disease associated with neoplasia. N Engl J Med 323: 1729-1735

Bedane C, Prost C, Thomine E, Intrator L, Joly P, Caux F, Blecker M, Bernard P, Leboutet MJ, Tron F, Lauret P, Bonnetblanc JM, Dubertret L (1996) Binding of autoantibodies is not restricted to desmosomes in pemphigus vulgaris: comparison of 14 cases of pemphigus vulgaris and 10 cases of pemphigus foliaceus studied by western immunoblot and immu-noelectron microscopy. Arch Dermatol Res 288: 343-352 Bhol K, Ahmed AR, Aoki V, Mohimen A, Nagarwalla N, Natarajan K (1995) Correlation of peptide specificity and IgG subclass with pathogenic and nonpathogenic autoantibodies in pemphigus vulgaris: a model for autoimmunity. Proc Natl Acad Sci USA92: 5239-5243 Brandsen R, Frusic-Zlotkin M, Lyubimov H, Yunes F, Michel B, Tamir A, Milner Y, Brenner S (1997) Circulating pemphigus IgG in families of patients with pemphigus: comparison of indirect immunofluorescence, direct immunofluorescence, and immunoblotting. J Am Acad Dermatol 36: 44-52 Brenner S, Bialy-Golan A, Anhalt GJ (1997) Recognition of pemphigus antigens in drug-induced pemphigus vulgaris and pemphigus foliaceus. J Am Acad Dermatol 36: 919-923 Delgado JC, Yunis EJ, Ahmed AR, Alper CA, Ahmad M Khan AA, Rehman SB, Rojas AI, Bhol K, Yunis JJ, Hameed A (1997) Pemphigus vulgaris autoantibody response is linked to HLA-DQß1*0503 in Pakistani patients. Hum Immunol 57: 110-119 Ding X, Fairley JA, Diaz LA, Lopez-Swiderski A, Mascaro JM Jr, Aoki V (1997). Mucosal and mucocutaneous (generalized) pemphigus vulgaris show distinct autoantibody profiles. J Invest Dermatol 109: 592-596 Emery DJ, Diaz LA, Fairly JA, Lopez A, Taylor AF, Giudice GJ (1995) Pemphigus foliaceus and pemphigus vulgaris autoantibodies react with the extracellular domain of desmog-lein 1. J Invest Dermatol 104: 323-328 Eming R, Rech J, Kalden JR, Schuler G, Harrer T, Hertl M. Removal of pathogenic autoanibo-

dies in patients with severe pemphigus by immunoadsorption. (in preparation) Esaki C, Kitajima Y, Osada K, Yamada T, Seishima M (1995) Pharmacologic evidence for involvement of phospholipase C in pemphigus IgG-induced inositol 1,4,5-trisphosphate generation, intracellular calcium increase, and plasminogen activator secretion in DJM-1 cells, a squamous cell carcinoma line. J Invest Dermatol 105: 329-333 Feliciani C, Toto P, Amerio P, Pour SM, Coscione G, Shivji G, Wang B, Sauder D (2000) In vitro and in vivo expression of interleukin-1alpha and tumor necrosis factor-alpha mRNA in pemphigus vulgaris: interleukin-1alpha and tumor necrosis factor-alpha are involved in acantholysis. J Invest Dermatol 114: 71-77 Fleischmann M, Dreno B, Litoux P, Bernard P, Celerier P (1996) Long-term interferon-alpha therapy induces autoantibodies against epidermis. Dermatology 192:50-55 Gomi H, Kawada A, Amagai M, Matsuo I (1999) Pemphigus erythematosus: detection of anti-desmoglein-1 antibodies by ELISA. Dermatology 199: 188-189 Hashimoto T, Shafran M, Webber PA, Lazarus GS, Singer KH (1983) Anti-cell surface pemphigus autoantibody stimulates plasminogen activator activity of human epidermal cells. A mechanism for the loss of epidermal cohesion and blister formation. J Exp Med 157:259-272

Hashimoto K, Hashimoto T, Hiashiyama M, Nishikawa T, Garrod DR, Yoshikawa K (1994) Detection of anti-desmocollin I and II autoantibodies in two cases of of Hallopeau type pemphigus vegetans by immunoblot analysis. J Dermatol Sci 7: 100-106 Hashimoto T, Amagai M, Watanabe K, Dmochowski M, Chidgey MAJ, Yue KKM, Garrod DR, Nishikawa T (1995a) A case of pemphigus vulgaris showing reactivity with pemphigus antigens (Dsg1 and Dsg3) and desmocollins. J Invest Dermatol 104: 541-544 Hashimoto T, Gamou S, Korman NJ, Boorsma DM, Stevens HP, Black MM, Bhogal BS, Chorzelski TP, Watanabe K, Amagai M (1995b) Characterization of paraneoplastic pemphigus autoantigens by immunoblot analysis. J Invest Dermatol 104: 829-834 Hashimoto T, Nishikawa T, Amagai M, Ishii K, Komori K, Kobayashi Y, Garrod DR, Chidgey MA, Miyasato M, Mori O, Kiyokawa C (1997) Human desmocollin 1 (Dsc1) is an autoantigen for the subcorneal pustular dermatosis type of IgA pemphigus. J Invest Dermatol 109: 127-131

Hertl M, Amagai M, Ishii K, Stanley J, Sundaram H, Katz SI (1998a) Recognition of desmog-lein 3 by autoreactive T cells in pemphigus vulgaris patients and normals. J Invest Dermatol 110: 62-66

Hertl M (1998b) Molecular characterization of autoantibodies in pemphigus: In Conrad K, Humbel RL, Meurer M, Shoenfeld Y and Tan EM (eds.): Pathogenic and diagnostic relevance of autoantibodies. Pabst Science Publishers, Berlin Hertl M, Riechers R (1999) Analysis of the T cells that are potentially involved in autoantibody production in pemphigus vulgaris. J Dermatol 26: 748-752 Hertl M (2000) Humoral and cellular autoimmunity in autoimmune bullous skin disorders. Int

Arch Allergy Immunol 122: 91-100 Huilgol SC, Black MM (1995) Management of the immunobullous disorders. II. Pemphigus.

Clin Exp Dermatol 20: 283-293 Ishii K, Amagai M, Komai A, Ebharas T, Chorzelski P, Nishikawa T, Hashimoto T (1998) Desmoglein 1 and desmoglein 3 as autoimmune target of herpetiform pemphigus. J Invest Dermatol 110: 510A

Iwatsuki KI, Harada H, Yokote R, Kaneko F (1995). Differences in the expression of pemphigus antigens during epidermal differentiation. Br J Dermatol 133: 209-216 Jablonska S, Chorzelski TP, Beutner EH, Chorzelska J (1975) Herpetiform pemphigus, a variable pattern of pemphigus. Int J Dermatol 14: 353-359 Jiao D, Bystryn JC (1997) Sensitivity of indirect immunofluorescence, substrate specificity, and immunoblotting in the diagnosis of pemphigus. J Am Acad Dermatol 37: 211-216 Joly P, Gilbert D, Thomine E, Delpech A, Verdier S, Lauret P, Tron F (1993) Immunofluore-sence and immunoelectron microscopy analyses of a human monoclonal anti-epithelial cell surface antibody that recognizes a 185-kD polypeptide: a component of the paraneoplastic pemphigus antigen complex. J Invest Dermatol 101: 339-345 Joly P, Tron F, Lauret P, Delpech A, Ghohestani R, Zitouni M, Thomine E, Gilbert D (1997) Identification of a new antibody population directed against a desmosomal plaque antigen in pemphigus vulgaris and pemphigus foliaceus. J Invest Dermatol 108: 469-475 Juhasz I, Lazarus GS, Murphy GF, Shih IM, Herlyn M (1993) Development of pemphigus vulgaris-like lesions in severe combined immunodeficiency disease mice with lymphocytes from patients. J Clin Invest 92: 2401-2407 Karpati S, Amagai M, Liu WL, Dmochowski M, Hashimoto T, Horvath A (2000) Identification of desmoglein 1 as autoantigen in a patient with intraepidermal neutrophilic IgA dermatosis type of IgA pemphigus. Exp Dermatol 9: 224-228 Kazerounian S, Mahoney MG, Uitto J, Aho S (2000) Envoplakin and periplakin, the paraneoplastic pemphigus antigens, are also recognized by pemphigus foliaceus autoantibodies. J Invest Dermatol 11: 505-507 Kim SC, Kwon YD, Lee IJ, Chang SN, Lee TG (1997) cDNA cloning of the 210-kD paraneoplastic pemphigus antigen reveals that envoplakin is a component of the antigen complex. J Invest Dermatol 109: 365-369 Kirsner RS, Anhalt GJ, Kerdel FA (1995) Treatment with alpha interferon associated with the development of drug-induced pemphigus. Br J Dermatol 132: 474-478 Kljuic A, Bazzi H, Sundberg JP, Martinez-Mir A, O'Shaughnessy R, Mahoney MG, Levy M, Montagutelli X, Ahmad W, Aita VM, Gordon D, Uitto J, Whiting D, Ott J, Fischer S, Gilliam TC, Jahoda CA, Morris RJ, Panteleyev AA, Nguyen VT, Christiano AM (2003) Desmoglein 4 in hair follicle differentiation and epidermal adhesion: evidence from inherited hypotrichosis and acquired pemphigus vulgaris. Cell 113:249-260 Koch PJ, Mahoney MG, Ishikawa H, Pulkkinen L, Uitto J, Shultz L, Murphy GF, Whitacker-Menezes D, Stanley JR (1997) Targeted disruption of the pemphigus vulgaris antigen (desmoglein 3) gene in mice causes loss of keratinocyte cell adhesion with a phenotype similar to pemphigus vulgaris. J Cell Biol 137: 1091-1102 Korman NJ, Eyre RW, Zone J, Stanley JR (1991a) Demonstration of an adhering-junction molecule (plakoglobin) in the autoantigens of pemphigus foliaceus and pemphigus vulgaris. N Engl J Med 321: 631-635 Korman NJ, Eyre RW, Zone J, Stanley JR (1991b) Drug-induced pemphigus: autoantibodies directed against the pemphigus antigen complexes are present in penicillamine- and captopril-induced pemphigus. J Invest Dermatol 96: 273-276 Kowalczyk AP, Green KJ, Stanley JR, Hashimoto T, Borgwardt JE, Anderson JE (1995) Pemphigus sera recognize conformationally sensitive epitopes in the amino-terminal region of desmoglein-1. J Invest Dermatol 105: 147-152

Kricheli D, David M, Frusic-Zlotkin M (2000) The distribution of pemphigus vulgaris IgG subclasses and reactivity with desmoglein 3 and 1 in pemphigus patients and first-degree relatives. B J Dermatol 143: 337-342 Kubo A, Yoshikawa K, Hashimoto K, Higashiyama M, Doi T, Hashimoto T, Amagai M (1997) Herpetiform pemphigus showing reactivity with pemphigus vulgaris antigen (desmoglein 3). Br J Dermatol 137: 109-113 Lever, WF (1953) Pemphigus. Medicine 32: 1-123

Lin MS, Swartz SL, Lopez A, Ding X, Fernandez-Vina MA, Stastny P, Fairley JA, Diaz L (1997) Development and characterization of desmoglein 3-specific T cells from patients with pemphigus vulgaris. J Clin Invest 99: 31-40 Lin MS, Fu CF, Aoki V, Hans-Filho G, Riviti EA, Moraes JR, Lazaro AM, Giugice GJ, Stastny P, Diaz LA (2000) Desmoglein 1-specific T lymphocytes from patients with endemic pemphigus foliaceus (fogo selvagem). J Clin Invest 105: 207-213 Meftl M, Stauber A, Mainka A, Klingel R, Schuler G, Hertl M (2003) Successful removal of pathogenic autoantibodies in pemphigus by immunoadsorption with a tryptophan-linked polyvinylalcohol adsorber. Br J Dermatol 149:598-605 Mahoney MG, Aho S, Uitto J, Stanley JR (1998) The members of the plakin family of proteins recognized by paraneoplastic pemphigus antibodies include periplakin. J Invest Dermatol 111: 308-313

Mahoney MG, Wang Z, Rothenberger KL, Koch PJ, Amagai M, Stanley JR (1999) Explanation for localization of blisters in pemphigus patients. J Clin Invest 103: 461-468 Matzner Y, Erlich HA, Brautbar C, Sanilevitch A, Landau M, Brenner S, Friedmann A (1995) Identical HLA class II alleles predispose to drug-triggered and idiopathic pemphigus vulgaris. Acta Derm Venereol 75: 12-14 Mutasim DF (1999) Autoimmune bullous diseases: diagnosis and management. Dermatol Nurs 11: 15-21

Nakaji S (2001) Current topics on immunoadsorption therapy. Ther Apher 5:301-305 Nguyen VT, Ndoye A, Shultz LD, Pittelkow MR, Grando SA (2000a) Antibodies against keratinocyte antigens other than desmogleins 1 and 3 can induce pemphigus vulgaris-like lesions. J Clin Invest 106: 1467-1479 Nguyen VT, Ndoye A, Grando SA (2000b) Pemphigus vulgaris antibody identifies pempha-xin. A novel keratinocyte annexin-like molecule binding acetylcholine. J Biol Chem 275: 29466-29476

Nguyen VT, Ndoye A, Grando SA (2000c) Novel human alpha9 acetylcholine receptor regulating keratinocyte adhesion is targeted by pemphigus vulgaris autoimmunity. Am J Pathol 157: 1377-1391

Niizeki H, Inamoto N, Nakamura K, Tsuchimoto K, Hashimoto T, Nishikawa T (1994) A case of pemphigus foliaceus after interferon-alpha-2a therapy. Dermatology 189: 129-130 Nishifuji K, Amagai M, Kuwana M, Iwasaki T, Nishikawa T (2000) Detection of antigen-specific B cells in patients with pemphigus vulgaris by enzyme-linked immunospot assay: requirement of T cell collaboration for autoantibody production. J Invest Dermatol 114: 88-94

Nishifuji K, Nagasaka T, Ota T, Whittock NV, Amagai M (2004) Is desmoglein 4 involved in blister formation in pemphigus or impetigo? J Invest Dermatol 122: A33 Nousari HC, Anhalt GJ (1999) Pemphigus and bullous pemphigoid. Lancet 354: 667-672 Ochsendorf FR, Schofer H, Milbradt R (1987) Pemphigus erythematosus - detection of anti-DNA antibodies. Hautarzt 1987 38: 400-403 Ohata Y, Amagai M, Ishii K, Hashimoto T (2001) Immunoreactivity against intracellular domains of desmogleins in pemphigus. J Dermatol Sci 25: 64-71 Ongenae KC, Temmerman LJ, Vermander F (1999) Intercellular Ig A dermatosis. Eur J Der-matol 9: 85-94

Oursler JR, Labib RS, Ariss-Abdo L, Burke T, O'Keefe EJ, Anhalt GJ (1992) Human autoantibodies against desmoplakins in paraneoplastic pemphigus. J Clin Invest 89: 1775-1782 Parodi A, Semino M, Gallo R, Rebora A (1993) Bullous eruption with circulating pemphiguslike antibodies following interferon-alpha therapy. Dermatology 186: 155-157 Proby C, Fujii Y, Owaribe K, Nishikawa T, Amagai M (1999) Human antibodies against HD1/plectin in paraneopolastic pemphigus. J Invest Dermatol 112: 153-156

Prost C, Intrator L, Wechsler J (1991) IgA autoantibodies bind to pemphigus vulgaris antigen in a case of intraepidermal neutrophilic IgA dermatosis. J Am Acad Dermatol 25: 846-848 Rädisch T, Riechers R, Hertl M (2002) The humanized SCID mouse model to study HLA class

II-linked autoimmunity to desmoglein 3 in pemphigus Br. J. Dermatol. 146: 189-193 Ramseur WL Richards F, Duggan DB (1989) A case of fatal pemphigus vulgaris in association with beta interferon and interleukin 2 therapy. Cancer 63: 2005-2007 Riechers R, Grötzinger A, Hertl M (1999) HLA class II restriction of autoreactive T cell responses in pemphigus vulgaris and potential application for a specific immunotherapy. Autoimmunity 30: 183-196 Santi CG, Maruta CW, Aoki V, Sotto MN, Rivitti EA, Diaz LA (1996) Pemphigus herpetiformis is a rare clinical expression of nonendemic pemphigus foliaceus, fogo selvagem, and pemphigus vulgaris. J Am Acad Dermatol 34: 40-46 Schmidt E, Klinker E, Opitz A, Herzog S, Sitaru C, Goebeler M, Mansouri, Taleghoni B, Brocker EB, Zillikens D (2003) Protein A immunoadsorption: a novel and effective adjuvant treatment of severe pemphigus. Br J Dermatol 148:1222-1229. Seishima M, Kitajima Y, Hashimoto T, Mori S, Osada K, Esaki C (1995) Pemphigus IgG, but not bullous pemphigoid IgG, causes a transient increase in intracellular calcium and inositol 1,4,5-triphosphate in DJM-1 cells, a squamous cell carcinoma line. J Invest Dermatol 104: 33-37

Senear FE, Usher B (1926) An unusual type of pemphigus combining features of lupus erythematosus. Arch Dermatol 13: 761-781 Shimizu H, Nishikawa T, Hashimoto T, Kikuchi A, Ishiko A, Masunaga T (1995) Pemphigus vulgaris and pemphigus foliaceus sera show an inversely graded binding pattern to extracellular regions of desmosomes in different layers of human epidermis. J Invest Der-matol 105: 153-159

Shirakata Y, Hashimoto K, Nishikawa T, Hanakawa Y, Amagai M (1998) Lack of mucosal involvement in pemphigus foliaceus may be due to low expression of desmoglein 1. J Invest Dermatol 110: 76-78

Sinha AA, Brautbar C, Szafer F, Friedmann A, Tzfoni E, Todd JA, Steinman L, McDevitt HO (1988) A newly characterized HLA-DQß allele associated with pemphigus vulgaris. Science 239: 1026-1029

Späth S, Riechers R, Borradori L, Zillikens D, Bdinger L, Hertl M (2001) Detection of autoantibodies of various subclasses (IgG1, IgG4, IgA, IgE) against desmoglein 3 in patients with acute onset and chronic pemphigus vulgaris. Br J Dermatol 144:1183-1188 Supapannachart N, Mutasim DF (1993) The distribution of IgA pemphigus antigen in human skin and the role of IgA anti-cell surface antibodies in the induction of intraepidermal acantholysis. Arch Dermatol 129: 605-608 Tremeau-Martinage C, Bazex J, Oksman F (1995) Immunoglobulin G subclass distribution of anti-intercellular substance antibodies in pemphigus. Ann Dermatol Venereol 122: 409-411

Veldman C, Stauber A, Wassmuth R, Uter W, Schuler G, Hertl M (2003) Dichotomy of auto-reactive Th1 and Th2 cell responses to desmoglein 3 in patients with pemphigus vulgaris (PV) and healthy carriers of PV-associated HLA class II alleles. J Immunol. 170: 635-42. Veldman C, Gebhard KL, Uter W, Wassmuth R, Grotzinger J, Schultz E, Hertl M (2004a) T cell recognition of desmoglein 3 peptides in patients with pemphigus vulgaris and healthy individuals. J Immunol 172:3883-3892. Veldman C, Hohne A, Dieckmann D, Schuler G, Hertl M (2004b) Type I regulatory T cells specific for desmoglein 3 are more frequently detected in healthy individuals than in patients with pemphigus vulgaris. J Immunol 172:6468-6475. Wallach J (1992) Intraepidermal IgA pustulosis. J Am Acad Dermatol 27: 993-1000 Wang J, Kwon J, Ding X, Fairley JA, Woodley DT, Chan LS (1997) Nonsecretory IgA1 autoantibodies targeting desmosomal component desmoglein 3 in intraepidermal neutro-philic IgA dermatosis. Am J Pathol 150: 1901-1906 Warren SJP, Lin MS, Giudice G, Hoffmann RG, Hans-Filho G, Aoki V, Rivitti EA, Dos Santos V, Diaz LA for the cooperative group of fogo selvagem research (2000) The prevalence of antibodies against desmoglein 1 in endemic pemphigus foliaceus in Brazil. N Engl J Med 343: 23-30

Whittock NV, Bower C (2003) Genetic evidence for a novel human desmosomal cadherin, desmoglein 4. J Invest Dermatol 120: 523-530 Wu H, Wang ZH, Yan A, Lyle S, Fakharzadeh S, Wahl JK, Wheelock MJ, Ishikawa H, Uitto J, Amagai M, Stanley JR (2000) Protection against pemphigus foliaceus by desmoglein 3 in neonates. N Engl J Med 343: 31-35 Wucherpfennig KW, Yu WB, Bhol K, Monos DS, Argyris E, Karr RW, Ahmed AR, Strominger JL (1995) Structural basis for major histocompatibility complex (MHC)-linked susceptibility to autoimmunity: charged residues of a single MHC binding pocket confer selective presentation of self-peptides in pemphigus vulgaris. Proc Natl Acad Sci USA 92: 11935-11939

Yasuda H, Kobayashi H, Hashimoto T, Itoh K, Yamane M, Nakamura J (2000) Subcorneal pustular dermatosis type of IgA pemphigus: demonstration of autoantibodies to desmo-collin-1 and clinical review. Br J Dermatol 143: 144-148

0 0

Post a comment